原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征诊治进展*

doi:10.3969/j.issn.1672-5069.2023.02.038

摘要/Abstract

摘要： 目的原发性胆汁性胆管炎(PBC)是一种慢性自身免疫性肝内胆汁淤积性疾病,自身免疫性肝炎(AIH) 是一种自身免疫反应介导的肝脏实质性炎症。该两种疾病共存于同一患者,称之为“原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征(PBC-AIH OS)”。PBC-AIH OS患病率低,较单纯PBC进展快、预后差。目前,其诊断标准的临床应用及治疗方案的选择仍有挑战性。本文对PBC-AIH OS的定义、流行病学、临床特征、诊断、治疗和预后的最新研究进展进行了综述,以期获得更深入的认识,为疾病的精准诊疗提供依据。

关键词: 原发性胆汁性胆管炎, 自身免疫性肝炎, 重叠综合征, 诊治

Abstract: Objective Primary biliary cholangitis(PBC)is characterized by a chronic autoimmune intrahepatic cholestasis,and autoimmune hepatitis (AIH) is a liver parenchymal inflammation mediated by autoimmune reaction. When the two entities coexist in a given patient, we call it "PBC-AIH overlap syndrome (PBC-AIH OS)". The prevalence of PBC-AIH OS is low, but it progresses faster and has a poorer prognosis. The clinical diagnosis and the choice of treatment are still challenging. Therefore, in this article, we reviewed the latest progress in the definition, epidemiology, clinical features, diagnosis, treatment and prognosis of patients with PBC-AIH OS, in order to help the appropriate diagnosis and treatment of the disease.

Key words: Primary biliary cholangitis, Autoimmune hepatitis, Overlap syndrome, Diagnosis, Treatment

马莉, 高丽丽, 段雪飞. 原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征诊治进展^*[J]. 实用肝脏病杂志, 2023, 26(2): 301-304.

Ma Li, Gao Lili, Duan Xuefei. Diagnosis and treatment of patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome[J]. Journal of Practical Hepatology, 2023, 26(2): 301-304.

参考文献

[1] Rust C, Beuers U. Overlap syndromes among autoimmune liver diseases. World J Gastroenterol, 2008, 14(21): 3368-3373.
[2] Czaja AJ. Cholestatic phenotypes of autoimmune hepatitis. Clin Gastroenterol Hepatol, 2014, 12(9): 1430-1438.
[3] Boberg KM, Chapman RW, Hirschfield GM, et al. Overlap syndromes: the International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue. J Hepatol, 2011, 54(2): 374-385.
[4] European Association for the Study of the Liver. EASLclinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol, 2017, 67(1): 145-172.
[5] Lyu T, Chen S, Li M, et al. Regional variation and temporal trend of primary biliary cholangitis epidemiology: a systematic review and meta-analysis. J Gastroenterol Hepatol, 2021, 36(6): 1423-1434.
[6] Cheung KS, Seto WK, Fung J, et al. Epidemiology andnatural history of primary biliary cholangitis in the Chinese: a territory-based study in Hong Kong between 2000 and 2015. Clin Transl Gastroenterol, 2017, 8(8): e116.
[7] Tanaka A, Mori M, Matsumoto K, et al. Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res, 2019, 49(8): 881-889.
[8] Floreani A, Franceschet I, Cazzagon N. Primary biliary cirrhosis: overlaps with other autoimmune disorders. Semin Liver Dis, 2014, 34(3): 352-360.
[9] Hu Z, Ni P, Fan X, et al. Past hepatitis B virus infection was not associated with poorer response or the UK-PBC risk score in ursodeoxycholic acid-treated patients with primary biliary cirrhosis. Eur J Gastroenterol Hepatol, 2019, 31(2): 277.
[10] 杨宁, 孙可帅, 田思远, 等. 伴自身免疫性肝炎特征的原发性胆汁性胆管炎的临床特征. 解放军医学杂志, 2021, 46(1): 36-41.
[11] Jiang Y, Xu BH, Rodgers B, et al. Characteristics and inpatient outcomes of primary biliary cholangitis and autoimmune hepatitis overlap syndrome. J Clin Transl Hepatol, 2021, 9(3): 392-398.
[12] 中国医师协会风湿免疫科医师分会自身抗体检测专业委员会, 国家风湿病数据中心, 国家皮肤与免疫疾病临床医学研究中心. 自身免疫性肝病相关自身抗体检测的临床应用专家共识. 中华内科杂志, 2021, 60(7): 619-625.
[13] Bauer A, Habior A. Detection of autoantibodies against nucleoporin p62 in Sera of patients with primary biliary cholangitis. Ann Lab Med, 2019, 39(3): 291-298.
[14] 党富涛, 唐映. 自身抗体在原发性胆汁性胆管炎中的诊断及预后价值. 临床肝胆病杂, 2020, 36(5): 1164-1166.
[15] Sebode M, Weiler-Normann C, Liwinski T, et al. Autoantibodies in autoimmune liver disease-clinical and diagnostic relevance. Front Immunol, 2018, 9: 609.
[16] Himoto T, Yoneyama H, Kurokohchi K, et al. Clinical significance of autoantibodies to p53 protein in patients with autoimmune liver diseases. Can J Gastroenterol, 2012, 26(3): 125-129.
[17] Nguyen HH, Shaheen AA, Baeza N, et al. Evaluation of classical and novel autoantibodies for the diagnosis of primary biliary cholangitis-autoimmune hepatitis overlap syndrome (PBC-AIH OS). PLoS One, 2018, 13(3): e0193960.
[18] Malik N, Venkatesh SK. Imaging of autoimmune hepatitis and overlap syndromes. Abdom Radiol (NY), 2017, 42(1): 19-27.
[19] Wu HM, Sheng L, Wang Q, et al. Performance of transient elastography in assessing liver fibrosis in patients with autoimmune hepatitis-primary biliary cholangitis overlap syndrome. World J Gastroenterol, 2018, 24 (6): 737-743.
[20] Lee BT, Wang Y, Yang A, et al. IgG: IgM ratios of liver plasma cells reveal similar phenotypes of primary biliary cholangitis with and without features of autoimmune hepatitis. Clin Gastroenterol Hepatol, 2021, 19(2): 397-399.
[21] Kuiper EM, Zondervan PE, van Buuren HR. Paris criteria are effective in diagnosis of primary biliary cirrhosis and autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol, 2010, 8(6): 530-534.
[22] Liu F, Pan ZG, Ye J, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome: simplified criteria may be effective in the diagnosis in Chinese patients. J Dig Dis, 2014, 15(12): 660-668.
[23] 张骏飞, 许何明, 宋海燕, 等. 自身免疫性肝炎-原发性胆汁性胆管炎重叠综合征: 巴黎标准诊断中国患者灵敏度低. 实用肝脏病杂志, 2019, 22(4): 537-540.
[24] Bonder A, Retana A, Winston DM, et al. Prevalence of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. Clin Gastroenterol Hepatol, 2011, 9(7): 609-612.
[25] Wang QX, Selmi C, Zhou XM, et al. Epigenetic considerations and the clinical reevaluation of the overlap syndrome between primary biliary cirrhosis and autoimmune hepatitis. J Autoimmun, 2013, 41: 140-145.
[26] Wang G, Tanaka A, Zhao H, et al. The Asian Pacific Association for the Study of the liver clinical practice guidance: the diagnosis and management of patients with autoimmune hepatitis. Hepatol Int, 2021, 15(2): 223-257.
[27] Mack CL, Adams D, Assis DN, et al. Diagnosis and management of autoimmune hepatitis in adults and children: 2019 practice guidance and guidelines from the American Association for the Study of Liver Diseases. Hepatology, 2020, 72(2): 671-722.
[28] Zhang W, De D, Mohammed KA, et al. New scoring classification for primary biliary cholangitis-autoimmune hepatitis overlap syndrome. Hepatol Commun, 2018, 2(3): 245-253.
[29] Montano-Loza AJ, Corpechot C. Definition and management of patients with primary biliary cholangitis and an incomplete response to therapy. Clin Gastroenterol Hepatol, 2021, 19(11): 2241-2251 e2241.
[30] Chazouilleres O, Wendum D, Serfaty L, et al. Long term outcome and response to therapy of primary biliary cirrhosis-autoimmune hepatitis overlap syndrome. J Hepatol, 2006, 44(2): 400-406.
[31] 杨宁, 田思远, 张苗, 等. 熊去氧胆酸联合糖皮质激素治疗伴自身免疫性肝炎特征原发性胆汁性胆管炎的效果分析. 解放军医药杂志, 2021, 33(3): 72-77.
[32] Martinez Casas OY, Diaz Ramirez GS, Marin Zuluaga JI, et al. Autoimmune hepatitis - primary biliary cholangitis overlap syndrome. Long-term outcomes of a retrospective cohort in a university hospital. Gastroenterol Hepatol, 2018, 41(9): 544-552.
[33] Yang F, Wang Q, Wang Z, et al. The natural history and prognosis of primary biliary cirrhosis with clinical features of autoimmune hepatitis. Clin Rev Allergy Immunol, 2016, 50(1): 114-123.
[34] Yoshioka Y, Taniai M, Hashimoto E, et al. Clinical profile of primary biliary cirrhosis with features of autoimmune hepatitis: Importance of corticosteroid therapy. Hepatol Res, 2014, 44(9): 947-955.

原发性胆汁性胆管炎-自身免疫性肝炎重叠综合征诊治进展^*

Diagnosis and treatment of patients with primary biliary cholangitis-autoimmune hepatitis overlap syndrome

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